“Systemic Lupus Erythematosus” Cutaneous features Iraj Salehi-Abari “Systemic Lupus Erythematosus” Cutaneous features Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir SLE

Definition: Chronic disease Systemic/Multi-organ disease Iraj Salehi-Abari Definition: Chronic disease Systemic/Multi-organ disease Inflammatory/Autoimmune disease Autoantibody/Immune Complex Skin, Joints, Kidneys, Lungs, Nervous system, Serous membranes,… y SLE

Cutaneous features: Skin involvement: > 80% Iraj Salehi-Abari Cutaneous features: Skin involvement: > 80% DD with all skin diseases Erythemato-desquamation Photosensitive: 60-100% Acute/subacute/chronic Localized/generalized SLE

Iraj Salehi-Abari Cutaneous features: SLE

Acute Cutaneous Lupus Erythematosus (ACLE) : Localized, “Malar rash”

Acute Cutaneous Lupus Erythematosus (ACLE) : Localized Iraj Salehi-Abari Acute Cutaneous Lupus Erythematosus (ACLE) : Localized Malar rash (“Butterfly rash”): 50% Usually after UV light exposure Mistaken for a sunburn Warm, edematous, peau d’ orange Sunscreens  enhance the redness of rash SLE

Malar rash (“Butterfly rash”): 50% Usually after UV light exposure Iraj Salehi-Abari Malar rash (“Butterfly rash”): 50% Usually after UV light exposure Mistaken for a sunburn Warm, edematous, peau d’ orange Sunscreens  enhance the redness of rash SLE

Acute Cutaneous Lupus Erythematosus (ACLE) : Generalized Iraj Salehi-Abari Acute Cutaneous Lupus Erythematosus (ACLE) : Generalized Maculopapular lupus rash Sun-exposed area type Erythrodermic type Bullous lupus rash Toxic epidermal necrolysis variant of SLE Periungual telangiectasia SLE

Subacute Cutaneous Lupus Erythematosus (SCLE): Psoriaform and/or annular lesions

Subacute Cutaneous Lupus Erythematosus (SCLE): Iraj Salehi-Abari Subacute Cutaneous Lupus Erythematosus (SCLE): There are two clinical forms: Papulosquamous (“Psoriasiform”) form “Annular” form Skin areas: Shoulder, forearms, neck and upper torso 50% of SCLE have SLE 10% of SLE have SCLE SLE

Chronic Cutaneous Lupus Erythematosus (CCLE): Iraj Salehi-Abari Chronic Cutaneous Lupus Erythematosus (CCLE): Discoid Lupus Erythematosus (“DLE”): A scaly plaque with follicular plugging, central scar and atrophy, telangiectasias and hyper/hypo-pigmentation Localized DLE: above the neck Generalized DLE: above and below the neck 10-15% of DLE  SLE Up to 25% of SLE  DLE SLE

Chronic Cutaneous Lupus Erythematosus (CCLE): Iraj Salehi-Abari Chronic Cutaneous Lupus Erythematosus (CCLE): Hypertrophic (verrucous) lupus Lupus panniculitis (profundus) Lupus erythematosus tumidus Chilblains lupus DLE/Lichen planus overlap SLE

Vascular skin lesions of SLE: Iraj Salehi-Abari Vascular skin lesions of SLE: Periungual erythema and telangiectasia Livedo reticularis Facial telangiectasia Raynaud phenomenon Petechia/purpura SLE

Vascular skin lesions of SLE: Iraj Salehi-Abari Vascular skin lesions of SLE: Microinfarcts of the tips of fingers and toes Splinter hemorrhages “Janeway lesion” “Osler’s nodes” Atrophie blanche Chilblain Lupus Erythematosus (CHLE) SLE

Vascular skin lesions of SLE: Iraj Salehi-Abari Vascular skin lesions of SLE: Microinfarcts of the tips of fingers and toes Splinter hemorrhages “Janeway lesion” “Osler’s nodes” Atrophie blanche Chilblain Lupus Erythematosus (CHLE) SLE

Iraj Salehi-Abari Photosensitivity: An abnormal dermal erythema response after exposure to ultraviolet (UV) radiation such as sunshine,… 60-100% of cases, UV-B > UV-A Blonde, blue-eyed, fair-skinned > Brunettes > Blacks SLE

Nail lesions: Nail pitting, ridging and onycholysis: 25% Iraj Salehi-Abari Nail lesions: Nail pitting, ridging and onycholysis: 25% Periungual telangiectasia and erythema Half-moon (lunula) redness: 20% SLE

Hair loss (Alopecia): Majority of patients, some time during illness Iraj Salehi-Abari Hair loss (Alopecia): Majority of patients, some time during illness One or more areas of scalp, eyebrows, eyelashes, beard and body hair Scarring alopecia of scalp: DLE Nonscarring alopecia: “Lupus hair” SLE

Iraj Salehi-Abari Hair loss (Alopecia): SLE

Mucousal lesions: Oral ulcer: painless, superficial erosion Iraj Salehi-Abari Mucousal lesions: Oral ulcer: painless, superficial erosion Nasal ulcer Labial DLE Other oral lesions: white plaques, silvery scarred lesion, areas of erythema, aphthous ulcer, vesicular lesions and gingivitis SLE

Iraj Salehi-Abari Mucousal lesions: SLE